actas dermosiliogr. 2017;108(8):779---794

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Actas Dermosifiliogr. 2017;108(8):779---794 CASE AND RESEARCH LETTERS Dermoscopy of Erythromelanosis Follicularis Faciei et Colli Dermatoscopia de erythromelanosis folicular faciei et colli Dear Editor: Erythromelanosis follicularis faciei et colli (EFFC) is a rare pigmentary disease of unknown etiopathogenesis typically affecting the face/neck of children or young adults, which is clinically characterized by the combination of bilat- eral/symmetrical brownish pigmentation and erythema, associated with more or less evident follicular plugging. 1 Of note, such a condition is often associated with ker- atosis pilaris on the arms and shoulders, thereby letting some authors speculate that EFFC could be a variant of this latter dermatosis. 1 Not uncommonly, EFFC is mistaken for other similar pigmentary/erythematous dermatoses involving the aforementioned districts, with consequent diagnostic errors/delays and prescription of inappropri- ate therapies. 1 Over the last few years, several studies have shown that dermoscopic examination may be use- ful to assist the diagnosis of general skin diseases. 2---5 We here describe for the first time the use of dermoscopy as a noninvasive diagnostic aid in a case of EFFC, com- paring its dermoscopic findings with those detectable in other conditions which classically enter into the differential diagnosis. A 33-year-old man presented with a 6-year history of progressively worsening, asymptomatic, reddish-brown pig- mentation associated with slight roughness on the cheeks, temples, lateral aspects of the nose, and frontal area (Fig. 1). Polarized light dermoscopic examination (carried out with DermLite DL3×10; 3Gen, San Juan Capistrano, CA, USA) revealed whitish scales and numerous whitish fol- licular keratotic plugs over a reddish-brown background; moreover, several gray-blue granules (peppering) were also evident in the perifollicular and interfollicular areas (Fig. 2). Histological examination showed slight orthokeratosis, fol- licular hyperkeratosis, increased basal layer pigmentation, perivascular and periadnexal lymphocytic infiltrate, and pig- mentary incontinence with dermal melanophages (Fig. 2b), thus leading to the diagnosis of EFFC. Topical tacalcitol was prescribed and the use of sunscreen was recommended, with significant improvement of the clinical picture after eight weeks. Dermoscopic findings seen in our instance of EFFC are related to the peculiar histological features which char- acterize this condition, 1,6 with follicular plugging, scaling, peppering, and reddish-brown background respectively cor- responding to hyperkeratotic hair follicles, orthokeratosis, pigmentary incontinence/dermal melanophages, and der- mal vasodilation/hyperpigmentation of the basal layer. 1,6 Such a dermoscopic picture is similar to that reported in a recent case of erythrosis pigmentosa peribuccalis, a pigmentary dermatosis presenting as brownish-red pigmen- tation and small papules around the mouth and nose, which displayed erythema, scaling, yellowish follicular keratotic plugs, and perifollicular grayish globules/dots. 7 These sim- ilarities are easily explained by the fact that both such disorders may share several histological features, so much so that they are considered to be part of the same condi- tion spectrum by some authors. 7 Interestingly, the detection of the above-mentioned dermocopic features might come in handy in the noninvasive distinction of EFFC from its main differential diagnoses as the latter typically show different features. In particular: lichen planus pigmentosus usu- ally displays diffuse, structureless, brownish pigmentation and/or fine/coarse, gray-blue/brown dots/globules 8 ; Riehl’s melanosis constantly features brownish pseudonetwork, gray dots/granules and telangiectatic vessels 9 ; poikilo- derma of Civatte commonly shows structureless brownish pigmentation and telangiectatic vessels, with or without whitish areas (personal observations); melasma typically presents light yellow-brown uniform patches, with or without dark brown patches and capillary network 10 ; ker- atosis pilaris rubra atrophicans faciei frequently displays whitish follicular plugs over a reddish background with or without telangiectatic vessels (personal observations); demodicidosis mainly shows the so-called ‘‘Demodex tails’’ (creamy/whitish gelatinous threads representing the pres- ence of the mite itself under magnification) protruding out of follicular openings, ‘‘Demodex follicular open- ings’’ (round and coarse follicular openings containing light brown/grayish plugs surrounded by an erythematous halo), erythema and whitish scaling 3 ; keratosis pilaris often fea- tures coiled/twisted hair embedded in the horny layer, sometimes associated with perifollicular erythema and vas- cular ectasia (keratosis pilaris rubra) 4 ; and follicular lichen planus reported to show follicular keratotic plugs without broken or twisted hairs. 4 In conclusion, this paper emphasizes that dermoscopy might be used as an auxiliary tool in the noninvasive dif- ferential diagnosis of EFFC. Further studies are obviously needed to confirm our preliminar observations.

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Actas Dermosifiliogr. 2017;108(8):779---794

CASE AND RESEARCH LETTERS

Dermoscopy ofErythromelanosis FollicularisFaciei et Colli

Dermatoscopia de erythromelanosis folicularfaciei et colli

Dear Editor:

Erythromelanosis follicularis faciei et colli (EFFC) is a rarepigmentary disease of unknown etiopathogenesis typicallyaffecting the face/neck of children or young adults, whichis clinically characterized by the combination of bilat-eral/symmetrical brownish pigmentation and erythema,associated with more or less evident follicular plugging.1

Of note, such a condition is often associated with ker-atosis pilaris on the arms and shoulders, thereby lettingsome authors speculate that EFFC could be a variant ofthis latter dermatosis.1 Not uncommonly, EFFC is mistakenfor other similar pigmentary/erythematous dermatosesinvolving the aforementioned districts, with consequentdiagnostic errors/delays and prescription of inappropri-ate therapies.1 Over the last few years, several studieshave shown that dermoscopic examination may be use-ful to assist the diagnosis of general skin diseases.2---5 Wehere describe for the first time the use of dermoscopyas a noninvasive diagnostic aid in a case of EFFC, com-paring its dermoscopic findings with those detectable inother conditions which classically enter into the differentialdiagnosis.

A 33-year-old man presented with a 6-year history ofprogressively worsening, asymptomatic, reddish-brown pig-mentation associated with slight roughness on the cheeks,temples, lateral aspects of the nose, and frontal area(Fig. 1). Polarized light dermoscopic examination (carriedout with DermLite DL3×10; 3Gen, San Juan Capistrano,CA, USA) revealed whitish scales and numerous whitish fol-licular keratotic plugs over a reddish-brown background;moreover, several gray-blue granules (peppering) were alsoevident in the perifollicular and interfollicular areas (Fig. 2).Histological examination showed slight orthokeratosis, fol-licular hyperkeratosis, increased basal layer pigmentation,perivascular and periadnexal lymphocytic infiltrate, and pig-mentary incontinence with dermal melanophages (Fig. 2b),thus leading to the diagnosis of EFFC. Topical tacalcitol wasprescribed and the use of sunscreen was recommended, withsignificant improvement of the clinical picture after eightweeks.

Dermoscopic findings seen in our instance of EFFC arerelated to the peculiar histological features which char-acterize this condition,1,6 with follicular plugging, scaling,peppering, and reddish-brown background respectively cor-responding to hyperkeratotic hair follicles, orthokeratosis,pigmentary incontinence/dermal melanophages, and der-mal vasodilation/hyperpigmentation of the basal layer.1,6

Such a dermoscopic picture is similar to that reportedin a recent case of erythrosis pigmentosa peribuccalis, apigmentary dermatosis presenting as brownish-red pigmen-tation and small papules around the mouth and nose, whichdisplayed erythema, scaling, yellowish follicular keratoticplugs, and perifollicular grayish globules/dots.7 These sim-ilarities are easily explained by the fact that both suchdisorders may share several histological features, so muchso that they are considered to be part of the same condi-tion spectrum by some authors.7 Interestingly, the detectionof the above-mentioned dermocopic features might come inhandy in the noninvasive distinction of EFFC from its maindifferential diagnoses as the latter typically show differentfeatures. In particular: lichen planus pigmentosus usu-ally displays diffuse, structureless, brownish pigmentationand/or fine/coarse, gray-blue/brown dots/globules8; Riehl’smelanosis constantly features brownish pseudonetwork,gray dots/granules and telangiectatic vessels9; poikilo-derma of Civatte commonly shows structureless brownishpigmentation and telangiectatic vessels, with or withoutwhitish areas (personal observations); melasma typicallypresents light yellow-brown uniform patches, with orwithout dark brown patches and capillary network10; ker-atosis pilaris rubra atrophicans faciei frequently displayswhitish follicular plugs over a reddish background withor without telangiectatic vessels (personal observations);demodicidosis mainly shows the so-called ‘‘Demodex tails’’(creamy/whitish gelatinous threads representing the pres-ence of the mite itself under magnification) protrudingout of follicular openings, ‘‘Demodex follicular open-ings’’ (round and coarse follicular openings containing lightbrown/grayish plugs surrounded by an erythematous halo),erythema and whitish scaling3; keratosis pilaris often fea-tures coiled/twisted hair embedded in the horny layer,sometimes associated with perifollicular erythema and vas-cular ectasia (keratosis pilaris rubra)4; and follicular lichenplanus reported to show follicular keratotic plugs withoutbroken or twisted hairs.4

In conclusion, this paper emphasizes that dermoscopymight be used as an auxiliary tool in the noninvasive dif-ferential diagnosis of EFFC. Further studies are obviouslyneeded to confirm our preliminar observations.

780 CASE AND RESEARCH LETTERS

Figure 1 Clinical examination shows irregular areas of reddish-brown pigmentation of the cheeks, temples, lateral aspects of the

nose, and frontal area (a and b).

Figure 2 Polarized light dermoscopic examination displays whitish scales and numerous follicular keratotic plugs over a reddish-

brown background; moreover, several perifollicular and interfollicular gray-blue granules (peppering) are also evident in the box

(a). Histology reveals findings consistent with a diagnosis of erythromelanosis follicularis faciei et colli, i.e. slight orthokeratosis,

follicular hyperkeratosis, increased basal layer pigmentation, perivascular and periadnexal lymphocytic infiltrate, and pigmentary

incontinence with dermal melanophages (hematoxylin and eosin stain 200×) (b).

Conflicts of interest

The authors declare that they have no conflicts of interest.

References

1. Silva RS, Fonseca JC, Obadia D. Case for diagnosis ery-thromelanosis follicularis faciei et colli. An Bras Dermatol.2010;85:923---5.

2. Errichetti E, Stinco G, Lacarrubba F, Micali G. Der-moscopy of Darier’s disease. J Eur Acad Dermatol Venereol.2016;30:1392---4.

3. Errichetti E, Stinco G. Dermoscopy in general dermatology: apractical overview. Dermatol Ther (Heidelb). 2016;6:471---507.

4. Panchaprateep R, Tanus A, Tosti A. Clinical, dermoscopic, andhistopathologic features of body hair disorders. J Am Acad Der-matol. 2015;72:890---900.

5. Errichetti E, Stinco G. Dermoscopy in differential diagnosisof palmar psoriasis and chronic hand eczema. J Dermatol.2016;43:423---5.

6. Patterson JW. Disease of cutaneous appendages. In: PattersonJW, editor. Weedon’s skin pathology. 4th ed. Philadelphia, PA:Elsevier; 2015. p. 499.

7. Luna PC, Abad ME, González VM, Vigovich F, Casas JG, Lar-ralde MAT Erythrosis pigmentosa peribuccalis in an adolescent:dermoscopic description and management. Pediatr Dermatol.2016;33:e65---8.

8. Güngör S, Topal IO, Göncü EK. Dermoscopic patterns in activeand regressive lichen planus and lichen planus variants: a mor-phological study. Dermatol Pract Concept. 2015;5:45---53.

9. Wang L, Xu AE. Four views of Riehl’s melanosis: clinical appear-ance, dermoscopy, confocal microscopy and histopathology. JEur Acad Dermatol Venereol. 2014;28:1199---206.

10. Li Y, Liu J, Sun QN. Characteristic dermoscopic features ofmelasma. Zhongguo Yi Xue Ke Xue Yuan Xue Bao. 2015;37:226---9.

CASE AND RESEARCH LETTERS 781

E. Errichetti,a,∗ S. Pizzolitto,b G. Stincoa

a Department of Experimental and Clinical Medicine,

Institute of Dermatology, University of Udine, Italyb Department of Pathology, Santa Maria della Misericordia

Hospital, Udine, Italy

∗ Corresponding author.E-mail address: [email protected] (E. Errichetti).

http://dx.doi.org/10.1016/j.adengl.2017.04.0101578-2190/© 2017 Elsevier Espana, S.L.U. and AEDV. All rights reserved.

Hyperkeratotic lesions on legs

Lesiones hiperqueratósicas en piernas

Dear Editor,

Skin hyperkeratosis is a common pathology in dermatolog-ical consultation. From localized pathology, such as cornsor common warts, to diseases with a more diffuse effectsuch as psoriasis and ichthyosis, diagnosis is usually easy, inresponse to the history and location of lesions. But some-times histological or analytical study is essential.

We report a 71-year-old male with multimorbidity (hyper-tension, diabetes mellitus, ischemic heart disease, mitralregurgitation, left bundle branch block, hepatic steatosis,fibrosing alveolitis, COPD, hypercholesterolemia, hyper-uricemia and glaucoma) who presented hyperkeratoticlesions on the legs (Fig. 1). The patient had worked ten years

Figure 1 Hyperkeratotic lesion on the legs.

in a photografic laboratory and twenty as carpenter. Thelesions were asymptomatic and he had them for more thantwenty years. They were located only on the front side ofthe legs and other skin was respected. Lesions were 2---6 mmin diameter and were very adherent. Some had peripheralreinforcement. The patient showed no other symptoms anddenied use of cosmetics or chemicals in the area. He hadnot suffered weight loss or anorexia. He had no history ofcontact dermatitis, burns or trauma. There was low actinicexposure in the area. No family member had similar injuries.

The patient had a history of working in a carpentry workshopfor more than twenty years.

With suspicion of perforating skin disease, actinic kerato-sis, or porokeratosis, we carried out a 4 mm punch of one ofthe lesions. Histology showed orthokeratotic papillomatosiswith focal lymphoid infiltrate in papillary dermis. The sam-ple showed no atypia. The pathology guided us to keratoticlesions with a ‘church spire’ pattern (Fig. 2). This pattern

Figure 2 Hemanotoxylin-eosin (×100). Orthokeratotic papil-

lomatosis with focal lymphoid infiltrate in papillary dermis and

church spire pattern.

suggests multiple diagnostic possibilities: arsenic poison-ing or tars, nutritional deficits (phrynoderma), digitatakeratosis, Hopf verruciformis acrokeratosis, or stucco ker-atosis. Chest radiography was normal but analytical researchshowed urinary arsenic levels of 317 �g/g creatinine (normallevels in occupationally exposed people <100 �g/g creat-inine). The patient was diagnosed with chronic arsenicpoisoning.

Arsenic poisoning has, as its main cause, ingestion ofcontaminated water.1 This is a significant public healthproblem in areas of South and Southeast Asia (India, China,Taiwan, Philippines, Thailand, Bangladesh) and the Ameri-cas (Argentina, Chile, Mexico, and the USA) where arsenicalproducts are detected either naturally or occasionallyby industrial waste. As a second etiologic factor there isworkplace exposure. Health damage results from inorganicarsenic (the toxic form) in pesticides, herbicides, miningand galvanized microchips. Occasionally wood preservativescontain arsenic derivatives.2 This could explain our case.